Deaths linked to rare brain disease?

A type of rare, degenerative brain disease - not unlike mad cow disease - may have claimed the lives of two county residents within the span of two months, according to local health officials.

Deaths of two people - one in January of this year and another in December 2012 - were labeled “probable cases” of Creutzfeldt-Jakob’s disease (CJD), according to Kim Crane, communicable disease and prevention supervisor for the Cleveland County Health Department.

Diseases like CJD are marked by abnormal proteins called prions that can develop in the brains of both humans and animals, according to the Centers for Disease Control and Prevention.

About 350 deaths related to CJD were reported in the U.S. during 2009, according to the CDC’s website.

Thirteen CJD-related deaths were reported by the NC Department of Health and Human Services in 2010, the most recent year in which stats for disease cases were available. None of the cases reported were related to mad cow disease.

Crane said she wasn't allowed to release any additional information about the individuals involved in both cases, such as age, gender, residency in the county and additional medical information.

Varying forms of the disease

CJD comes in many forms, but two are most prominent: variant and classic.

Variant, or vCJD, has been linked to outbreaks of people eating infected beef from animals with bovine spongiform encephalopathy (BSE), also known as “mad cow disease.” Cases of variant CJD and BSE appeared almost simultaneously in the United Kingdom during the early 1990s, according to the Mayo Clinic’s website.

In 1996, three cases of CJD were identified in the US, but they were contracted in the United Kingdom and Saudi Arabia, according to Marilyn Haskell, a public health veterinarian and epidemiologist with the N.C. Department of Health and Human Services.

Classic CJD occurs spontaneously in humans from abnormal brain prions. It is not related to mad cow disease, Haskell said.

Classic CJD usually develops later in life around age 60, the CDC reports, while the average age of people contacting CJD linked to mad cow disease is about 28 years old.

“Having a probable case does not mean the person had CJD,” Haskell said.

No other probable or confirmed cases of CJD were reported on record in the county, Crane said.

Confirming presence of the disease

Generally, CJD carries symptoms similar to other brain disorders such as advanced Alzheimer’s, including dementia, memory loss, difficulty speaking and personality changes.

A person can have classic CJD for years before symptoms surface, and classic forms are fatal.

CJD can only be confirmed by a human brain autopsy or biopsy after death, according to health officials.

An autopsy wasn’t performed on either of the two probable cases in Cleveland County, Crane said.

Autopsies to confirm CJD in North Carolina have to be arranged by the hospital handling the case, Haskell said.

There is no known prevention or cure for classic CJD.

The disease can also be inherited through genetics or contaminated brain tissue. Passing contaminated tissues can occur in medical procedures, including cornea, skin transplants and even surgeries with contaminated instruments, the Mayo Clinic reports.

The National Institute of Neurological Disorders and Stroke says forms of CJD can’t be passed on through the air or by touching.